Invited review free radicals in disease processes: a compilation of cause and consequence
JMC Gutteridge, B Halliwell - Free radical research …, 1993 - Taylor & Francis
During the past forty years free radicals have been implicated in a large number of diseases,
often with the unspoken assumption they substantially contribute to the primary cause of the …
often with the unspoken assumption they substantially contribute to the primary cause of the …
Decreased proteolysis caused by protein aggregates, inclusion bodies, plaques, lipofuscin, ceroid, and 'aggresomes' during oxidative stress, aging, and disease
T Grune, T Jung, K Merker, KJA Davies - The international journal of …, 2004 - Elsevier
Protein aggregation seems to be a common feature of several neurodegenerative diseases
and to some extent of physiological aging. It is not always clear why protein aggregation …
and to some extent of physiological aging. It is not always clear why protein aggregation …
Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses
The neuronal ceroid lipofuscinoses (NCLs) are clinically and genetically heterogeneous
neurodegenerative disorders. Most are autosomal recessively inherited. Clinical features …
neurodegenerative disorders. Most are autosomal recessively inherited. Clinical features …
Cathepsin D deficiency induces lysosomal storage with ceroid lipofuscin in mouse CNS neurons
Cathepsin D-deficient (CD−/−) mice have been shown to manifest seizures and become
blind near the terminal stage [approximately postnatal day (P) 26]. We therefore examined …
blind near the terminal stage [approximately postnatal day (P) 26]. We therefore examined …
Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease)
In cathepsin D-deficient (CD−/−) and cathepsins B and L double-deficient (CB−/− CL−/−)
mice, abnormal vacuolar structures accumulate in neurons of the brains. Many of these …
mice, abnormal vacuolar structures accumulate in neurons of the brains. Many of these …
Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease)
The ceroid‐lipofuscinoses (Batten disease) are neurodegenerative inherited lysosomal
storage diseases of children and animals. A common finding is the occurrence of fluorescent …
storage diseases of children and animals. A common finding is the occurrence of fluorescent …
The neuronal ceroid-lipofuscinoses
M Haltia - Journal of Neuropathology & Experimental Neurology, 2003 - academic.oup.com
The neuronal ceroid-lipofuscinoses (NCLs) collectively constitute the most common group of
neurodegenerative diseases in childhood and usually show an autosomal recessive mode …
neurodegenerative diseases in childhood and usually show an autosomal recessive mode …
[HTML][HTML] The neuronal ceroid-lipofuscinoses: from past to present
M Haltia - Biochimica Et Biophysica Acta (BBA)-Molecular Basis …, 2006 - Elsevier
The neuronal ceroid-lipofuscinoses (NCLs) are inherited lysosomal storage diseases and
constitute the most common group of children's progressive encephalopathies. Most …
constitute the most common group of children's progressive encephalopathies. Most …
Age-related changes in protein oxidation and proteolysis in mammalian cells
T Grune, R Shringarpure, N Sitte… - The Journals of …, 2001 - academic.oup.com
Reactive oxygen species generated as by-products of oxidative metabolism, or from
environmental sources, frequently damage cellular macromolecules. Proteins are …
environmental sources, frequently damage cellular macromolecules. Proteins are …
Autophagy–physiology and pathophysiology
Abstract “Autophagy” is a highly conserved pathway for degradation, by which wasted
intracellular macromolecules are delivered to lysosomes, where they are degraded into …
intracellular macromolecules are delivered to lysosomes, where they are degraded into …